Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis
- Joseph F Merola, MD, MMSc, FAAD, FACR
Joseph F Merola, MD, MMSc, FAAD, FACR
- Assistant Professor, Harvard Medical school
- Department of Dermatology
- Department of Medicine, Division of Rheumatology
- Brigham and Women's Hospital
Sweet syndrome (acute febrile neutrophilic dermatosis) is a neutrophilic dermatosis characterized by the abrupt appearance of edematous and erythematous papules, plaques, or nodules on the skin. Fever, leukocytosis, and internal organ involvement can also occur. Sweet syndrome has been associated with infection, malignancy, pregnancy, and drug exposure. (See "Sweet syndrome (acute febrile neutrophilic dermatosis): Pathogenesis, clinical manifestations, and diagnosis".)
Systemic glucocorticoid therapy is a first-line treatment for Sweet syndrome, and usually results in dramatic clinical improvement. Colchicine, dapsone, and potassium iodide are additional effective therapies.
The management and prognosis of Sweet syndrome will be discussed here. Information on the clinical features and diagnosis of Sweet syndrome and an overview of other neutrophilic dermatoses are available separately. (See "Sweet syndrome (acute febrile neutrophilic dermatosis): Pathogenesis, clinical manifestations, and diagnosis" and "Neutrophilic dermatoses".)
APPROACH TO TREATMENT
Although a wide variety of therapeutic interventions have been utilized for Sweet syndrome, there is a paucity of high quality data on the treatment options. Support for the use of therapies for Sweet syndrome is primarily based on case series and individual reports of clinical experience. Systemic therapies, particularly systemic glucocorticoids, are the mainstays of treatment [1-3].
Although spontaneous resolution after weeks to months occurs in an unknown proportion of patients with classical Sweet syndrome, the prominent symptoms and the unpredictable disease course lead us to initiate treatment in most patients. In addition, although our experience indicates that drug-induced Sweet syndrome can improve within several weeks after withdrawal of an offending medication, we have found pharmacologic therapy useful for accelerating clinical improvement in these patients .
- Halpern J, Salim A. Pediatric sweet syndrome: case report and literature review. Pediatr Dermatol 2009; 26:452.
- Uihlein LC, Brandling-Bennett HA, Lio PA, Liang MG. Sweet syndrome in children. Pediatr Dermatol 2012; 29:38.
- Rochet NM, Chavan RN, Cappel MA, et al. Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients. J Am Acad Dermatol 2013; 69:557.
- Cohen PR, Kurzrock R. Sweet's syndrome: a review of current treatment options. Am J Clin Dermatol 2002; 3:117.
- O'Connor Reina C, Garcia Iriarte MT, Rodriguez Diaz A, et al. Tonsil cancer and Sweet's syndrome. Otolaryngol Head Neck Surg 1998; 119:709.
- Cohen PR, Holder WR, Tucker SB, et al. Sweet syndrome in patients with solid tumors. Cancer 1993; 72:2723.
- von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31:535.
- Mahajan VK, Sharma NL, Sharma RC. Sweet's syndrome from an Indian perspective: a report of four cases and review of the literature. Int J Dermatol 2006; 45:702.
- Zamanian A, Ameri A. Acute febrile neutrophilic dermatosis (Sweet's syndrome): a study of 15 cases in Iran. Int J Dermatol 2007; 46:571.
- Bourke JF, Keohane S, Long CC, et al. Sweet's syndrome and malignancy in the U.K. Br J Dermatol 1997; 137:609.
- Suehisa S, Tagami H. Treatment of acute febrile neutrophilic dermatosis (Sweet's syndrome) with colchicine. Br J Dermatol 1981; 105:483.
- Suehisa S, Tagami H, Inoue F, et al. Colchicine in the treatment of acute febrile neutrophilic dermatosis (Sweet's syndrome). Br J Dermatol 1983; 108:99.
- Maillard H, Leclech C, Peria P, et al. Colchicine for Sweet's syndrome. A study of 20 cases. Br J Dermatol 1999; 140:565.
- Sanchez MR. Miscellaneous treatments: thalidomide, potassium iodide, levamisole, clofazimine, colchicine, and D-penicillamine. Clin Dermatol 2000; 18:131.
- Sullivan TP, King LE Jr, Boyd AS. Colchicine in dermatology. J Am Acad Dermatol 1998; 39:993.
- Schiff BL, Kern AB, Bercovitch L. Sweet's syndrome: report of two atypical cases. Postgrad Med 1982; 71:55.
- el Sherif AI, Bharija SC, Belhaj MS, Singh G. Dapsone in Sweet syndrome. Int J Dermatol 1990; 29:737.
- Sitjas D, Puig L, Cuatrecasas M, De Moragas JM. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Int J Dermatol 1993; 32:261.
- Aram H. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Response to dapsone. Arch Dermatol 1984; 120:245.
- Horio T, Danno K, Okamoto H, et al. Potassium iodide in erythema nodosum and other erythematous dermatoses. J Am Acad Dermatol 1983; 9:77.
- Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007; 2:34.
- Case JD, Smith SZ, Callen JP. The use of pulse methylprednisolone and chlorambucil in the treatment of Sweet's syndrome. Cutis 1989; 44:125.
- Khatri ML, Taha M. Sweet's syndrome associated with myelodysplastic syndrome presenting as periorbital cellulitis. Int J Dermatol 2007; 46:496.
- Altomare G, Capella GL, Frigerio E. Sweet's syndrome in a patient with idiopathic myelofibrosis and thymoma-myasthenia gravis-immunodeficiency complex: efficacy of treatment with etretinate. Haematologica 1996; 81:54.
- von den Driesch P, Steffan C, Zöbe A, Hornstein OP. Sweet's syndrome--therapy with cyclosporin. Clin Exp Dermatol 1994; 19:274.
- Bourke JF, Berth-Jones J, Graham-Brown RA. Sweet's syndrome responding to cyclosporin. Br J Dermatol 1992; 127:36.
- Walling HW, Snipes CJ, Gerami P, Piette WW. The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Arch Dermatol 2006; 142:57.
- Browning CE, Dixon JE, Malone JC, Callen JP. Thalidomide in the treatment of recalcitrant Sweet's syndrome associated with myelodysplasia. J Am Acad Dermatol 2005; 53:S135.
- Kluger N, Gil-Bistes D, Guillot B, Bessis D. Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret®) in a case of refractory Sweet's syndrome. Dermatology 2011; 222:123.
- Seminario-Vidal L, Guerrero C, Sami N. Refractory Sweet's syndrome successfully treated with rituximab. JAAD Case Rep 2015; 1:123.
- Hashemi SM, Fazeli SA, Vahedi A, Golabchifard R. Rituximab for refractory subcutaneous Sweet's syndrome in chronic lymphocytic leukemia: A case report. Mol Clin Oncol 2016; 4:436.
- Agarwal A, Barrow W, Selim MA, Nicholas MW. Refractory Subcutaneous Sweet Syndrome Treated With Adalimumab. JAMA Dermatol 2016; 152:842.
- Lin CH, Yeh SP, Lin TY. Azacitidine is effective for the treatment of myelodysplastic syndrome and accompanied Sweet syndrome. Ann Hematol 2015; 94:1925.
- Jeanfils S, Joly P, Young P, et al. Indomethacin treatment of eighteen patients with Sweet's syndrome. J Am Acad Dermatol 1997; 36:436.
- Saxe N, Gordon W. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Four case reports. S Afr Med J 1978; 53:253.
- Kim MJ, Choe YH. EPONYM. Sweet syndrome. Eur J Pediatr 2010; 169:1439.
- SWEET RD. AN ACUTE FEBRILE NEUTROPHILIC DERMATOSIS. Br J Dermatol 1964; 76:349.
- Fett DL, Gibson LE, Su WP. Sweet's syndrome: systemic signs and symptoms and associated disorders. Mayo Clin Proc 1995; 70:234.
- Wawrzycki B, Chodorowska G, Pietrzak A, et al. Recurrent skin eruption in patient with chronic lymphocytic leukemia and lymphocytic infiltrates of the dermis resembling Sweet's syndrome. G Ital Dermatol Venereol 2011; 146:487.
- Choi HJ, Chang SE, Lee MW, et al. A case of recurrent Sweet's syndrome in an 80-year-old man: a clue to an underlying malignancy. Int J Dermatol 2006; 45:457.